A case of Hand-Schüller-Christian disease was reported.
A 43-year-old woman was well until she began to feel right coxalgia and noticed the xanthomatous dermatitis in her left upper eyelid in 1976.
In December 1976, a soft nodule was palpable on her right groin. An exploratory operation revealed it to be pyoid exudate from right iliac bone.
Curettage and drainage were repeated. But the exacerbation of the bone lesion occured with the enlargement of the skin lesion, and painless loss of all teeth.
In January 1979, a fistula was developed on the right iliac crest and massive bloody exudate was found.
She was hospitalized because of fever, anemia and cardiac failure.
The skin biopsy revealed abnormal proliferation of histiocytes and a diagnosis of histiocytosis-X (Hand-Schüller-Christian type) was made.
Bone X ray and bone scanning showed generalized osteolytic lesions, and chest X ray and echocardiogram revealed the presence of massive pericardial effusion. Pericardocentesis was performed and many foam cells were found.
KM 2210 was administered with marked improvement for bone lesions, partial regression of skin lesions but no effect on the pericardial effusion.
This is a very interesting case of Hand-Schüller-Christian disease, from the standpoint of the late onset, the very rare pericardial involvement and the responses to therapy. A review of the pertinent literature was included.
